ANSWER TO PHOTO QUIZ Philip A. Mackowiak, Section Editor A Homeless Man with Maculopapular Rash Who Died in Marseille, France (See page 1412 for Photo Quiz) Figure 1. A, Maculopapular erythema on the trunk. B, Eschar at the site of a tick bite on the right shoulder. Diagnosis: Mediterranean spotted fever (MSF) in its malignant form. At admission to the infectious and tropical diseases department at one of our institutions (Hôpital Nord, Marseille, France), physical examination revealed, in addition to the maculopapular rash and the eschar on the right shoulder (figure 1), several tiny black spots. Careful examination showed that these spots were actually ticks (a total of 22) attached on the skin. They were immediately removed from the patient’s skin (on the legs, groin, arms, and axilla). The patient had spent a total of 25 h in the emergency department, during which time he was examined by a medical student, a resident, and the attending physician; blood samples were drawn by a nurse; and he was directly supported by 3 people during performance of a spinal tap. Dermatologic examination was woefully incomplete, as often occurs when unhygienic, homeless, and/or drunken patients present to emergency units. All ticks were identified as Rhipicephalus sanguineus, the brown dog tick, including 1 female and nymphs (figure 2). Because MSF in its malignant form was suspected, empirical treatment with doxycycline and ciprofloxacin was started immediately. Six days after the patient’s death, blood culture for Rickettsiae (shellvial cell culture) became positive for Rickettsia conorii, the agent of MSF. The biopsy sample of the eschar (“tache noire”) (figure 1B) was positive for the same rickettsia by culture as well as by molecular methods. After autopsy, R. conorii DNA was amplified from a specimen of the spleen. Two of the 22 ticks tested positive for rickettsia, including 1 for R. conorii and 1 for Rickettsia Bar29 (a rickettsia of unknown pathogenicity, also associated with R. sanguineus) by molecular methods and shellvial cell culture. MSF due to R. conorii is endemic in the Mediterranean area, where it is transmitted by the brown dog tick, R. sanguineus. This tick is highly host-specific and rarely bites humans in France [1, 2]. As a result, despite the fact that these ticks live in the environment of dogs (i.e., close to humans), cases of MSF are sporadic and the incidence is relatively low. The case presented here is highly unusual in regard to the intensity of parasitism by R. sanguineus. It is the first time that we have observed 11 R. sanguineus feeding on a human body [1]. August 2003 was the hottest summer in the past 50 years in France. An exceptional number of days (117) had a temperature of 135 C recorded in southern France [3]. In these conditions, the biological cycle of the ticks might have changed, as well as their host-seeking and feeding behaviors [4, 5, 6]. The onset of MSF is abrupt and typical cases present with high fever, flulike symptoms, a black eschar (tache noire) at the tick bite site, and a maculopapular rash (figure 1) [7]. ANSWER TO PHOTO QUIZ • CID 2004:38 (15 May) • 1493 Figure 2. A, Rhipicephalus sanguineus female attached to the skin. B, Rhipicephalus sanguineus nymphs near the axilla. Although MSF is often considered a benign disease, severe forms may occur including major neurological manifestations and multiorgan involvement [8]. The mortality rate of MSF is usually estimated to be ∼2.5%, but a recent report has found a mortality rate of 32.3% among hospitalized patients in Portugal [10]. Classic risk factors for severe forms include advanced age, immunocompromise, chronic alcoholism and glucose-6phosphate-dehydrogenase deficiency, prior prescription of an inappropriate antibiotic, or delay of treatment [9, 11]. Fatal outcome has also been associated with diabetes, vomiting, dehydration, and uremia [10]. As in the case we describe, thrombocytopenia, abnormal hepatic function, and hyponatremia are common findings in severe MSF [7, 10]. Other frequent abnormal findings include elevated adenosine deaminase and triglyceride levels and abnormal urinalysis findings [7]. Treatment of MSF should never be delayed, and first-line therapy is doxycycline [2]. Marion Hemmersbach-Miller,1 Philippe Parola,1,2 Didier Raoult,2 and Philippe Brouqui,1,2 3. 4. 5. 6. 7. 8. 9. 10. 1 Service de Maladies Infectieuses et Tropicales, Hôpital Nord, AP-HM, and 2Unité des Rickettsies, Faculté de Médecine, Université de la Méditerranée, Marseille, France References 1. Gilot B, Laforge ML, Pichot J, Raoult D. Relationships between the Rhipicephalus sanguineus complex ecology and Mediterranean spotted fever epidemiology in France. Eur J Epidemiol 1990; 6:357–62. 2. Parola P, Raoult D. Ticks and tickborne bacterial diseases in humans: 1494 • CID 2004:38 (15 May) • ANSWER TO PHOTO QUIZ 11. an emerging infectious threat. Clin Infect Dis 2001; 32:897–928 (erratum: Clin Infect Dis 2001; 33:749). La chronologie des temperatures minimales et maximales, moyennies sur la France. Available at: http://www.meteo.fr/meteonet/actu/ dossiers/canicule_courtier/temp_quo_france.htm. Accessed 30 November 2003. Sonenshine D. Biology of ticks. New York: Oxford University Press, 1993. Raoult D, Tissot DH, Caraco P, Brouqui P, Drancourt M, Charrel C. Mediterranean spotted fever in Marseille: descriptive epidemiology and the influence of climatic factors. Eur J Epidemiol 1992; 8:192–7. Peter O, Burgdorfer W, Aeschlimann A, Chatelanat P. Rickettsia conorii isolated from Rhipicephalus sanguineus introduced into Switzerland on a pet dog. Z Parasitenkd 1984; 70:265–70. Anton E, Font B, Munoz T, Sanfeliu I, Segura F. Clinical and laboratory characteristics of 144 patients with Mediterranean spotted fever. Eur J Clin Microbiol Infect Dis 2003; 22:126–8. Raoult D, Gallais H, Ottomani A et al. Malignant form of Mediterranean boutonneuse fever: 6 cases [in French]. Presse Med 1983; 12: 2375–8. Raoult D, Zuchelli P, Weiller PJ, et al. Incidence, clinical observations and risk factors in the severe form of Mediterranean spotted fever among patients admitted to hospital in Marseilles 1983–1984. J Infect 1986; 12:111–6. de Sousa R, Nobrega SD, Bacellar F, Torgal J. Mediterranean spotted fever in Portugal: risk factors for fatal outcome in 105 hospitalized patients. Ann N Y Acad Sci 2003; 990:285–94. Amaro M, Bacellar F, Franca A. Report of eight cases of fatal and severe Mediterranean spotted fever in Portugal. Ann N Y Acad Sci 2003; 990: 331–43. Reprints or correspondence: Dr. Philippe Brouqui, Unité des Rickettsies, CNRS UMR 6020, IFR 48, Faculté de Médicine, Université de la Mediterranée, 27 Blvd. Jean Moulin, 13385 Marseille, Cedex 05, France ([email protected]). Clinical Infectious Diseases 2004; 38:1493–4 2004 by the Infectious Diseases Society of America. All rights reserved. 1058-4838/2004/3810-0026$15.00
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